Affordable Access

Access to the full text

Autoimmune and inflammatory manifestations occur frequently in primary immunodeficiencies

Authors
  • Alain Fischer
  • Provot, Johan
  • Jais, Jean-Philippe
  • Alcais, Alexandre
  • Mahlaoui, Nizar
  • members of the, Ceredih French P. I. D. ...
Type
Published Article
Journal
Journal of Allergy and Clinical Immunology
Publisher
Elsevier
Publication Date
Jul 20, 2017
Volume
140
Issue
5
Pages
1388–139300000000
Identifiers
DOI: 10.1016/j.jaci.2016.12.978
PMID: 28192146
Source
USPC - SET - SVS
Keywords
License
Green

Abstract

Background: Primary immunodeficiencies (PIDs) are inherited diseases associated with a considerable increase in susceptibility to infections. It is known that PIDs can also predispose to cancer and immune diseases, including allergy, autoimmunity, and inflammation. Objective: We aimed at determining the incidence of autoimmunity and inflammation in patients with PIDs. Methods: We have retrospectively screened 2183 consecutive cases of PID in the Centre de Référence Déficits Immunitaires Héréditaires registry (CEREDIH; the French national PID registry) for the occurrence of autoimmunity and inflammation. Results: One or more autoimmune and inflammatory complications were noted in 26.2% of patients, with a risk of onset throughout the patient's lifetime. The risk of autoimmune cytopenia was at least 120 times higher than in the general population, the risk of inflammatory bowel disease in children was 80 times higher, and the risk of other autoimmune manifestations was approximately 10 times higher. Remarkably, all types of PIDs were associated with a risk of autoimmune and inflammatory complications, although the greatest risk was associated with T-cell PIDs and common variable immunodeficiency. The occurrence of autoimmune disease is a negative prognostic factor for survival. Conclusions: Our results provide the basis for a detailed prospective evaluation of autoimmunity and inflammation in the context of PIDs, with a view to accurately assessing these risks and describing the possible effect of medical intervention.

Report this publication

Statistics

Seen <100 times